Terrill P J, Mayou B J, McKee P H, Eady R A
Department of Plastic Surgery, St. Thomas' Hosptial, London.
Br J Plast Surg. 1992 Aug-Sep;45(6):426-34. doi: 10.1016/0007-1226(92)90206-d.
Fifty patients with Dystrophic Epidermolysis Bullosa (DEB) underwent surgery including release of limb, oral, anal, eye and penile contractures and treatment of chronic skin ulceration or skin tumours. Correction of contractures involves extensive release of skin and underlying tissues, with split skin grafting of secondary defects. Specific regions are discussed. Recurrence is inevitable due to ongoing disease; however, functional improvement is obtained for several years. Management of chronic skin ulceration with split skin grafting has failed to produce long term healing, with local flaps successful but limited by the problem of donor site instability. Nine of the 17 patients over 20 years of age developed squamous cell carcinomas (29 lesions), benign hyperkeratosis (9) or malignant melanoma (1) requiring excision and skin grafting or amputation of digits. Local recurrence was infrequent (3 squamous cell carcinomas), with distant metastatic spread occurring in 1 patient.
50例营养不良性大疱性表皮松解症（DEB）患者接受了手术，包括松解肢体、口腔、肛门、眼部和阴茎挛缩，以及治疗慢性皮肤溃疡或皮肤肿瘤。挛缩的矫正包括广泛松解皮肤和深层组织，并对继发性缺损进行中厚皮片移植。文中讨论了特定区域。由于疾病持续存在，复发不可避免；然而，功能改善可持续数年。采用中厚皮片移植治疗慢性皮肤溃疡未能实现长期愈合，局部皮瓣移植成功，但受供区不稳定问题限制。17例20岁以上患者中有9例发生了鳞状细胞癌（29个病灶）、良性角化过度（9个）或恶性黑色素瘤（1个），需要进行切除、皮肤移植或手指截肢。局部复发不常见（3例鳞状细胞癌），1例患者发生远处转移。
